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Background: Midaortic syndrome (MAS) is a rare congenital or acquired condition marked by segmental or diffuse stenosis of the distal thoracic and/or abdominal aorta and its branches. The optimal approach to medical or interventional management of MAS and long-term outcomes in adults are not well defined. We reviewed MAS cases to characterize the natural history of aortic disease, identify prognostic factors, and evaluate the durability of invasive interventions., Methods: We conducted a retrospective review of patients with MAS who presented to Memorial Hermann Hospital and Baylor College of Medicine between 1997 and 2018. We categorized cases according to demographic and clinical manifestations, etiologies, the extent of aortic involvement, interventions, and vascular outcomes., Results: We identified a cohort of 13 patients with MAS. The etiology of MAS was identified in 6 cases, including genetic syndromes (neurofibromatosis type 1 (2/13), Williams syndrome (1/13), fibromuscular dysplasia (2/13), and Takayasu arteritis (1/13)). Mean age at first documented clinical event was 25.2 (2-67) years, but cases with genetic etiologies presented significantly younger (18.2 years). The most common primary anatomic site was the suprarenal and infrarenal aorta (zones 5-8). Extra-aortic locations involved the renal (4/13), celiac (3/13), and superior mesenteric (3/13) arteries. Clinical manifestations included hypertension (13/13), claudication (9/13), and postprandial abdominal pain (5/13). All patients with available follow-up data underwent at least one surgical or endovascular intervention (range: 1-8). Postoperative complications included renal failure requiring postdischarge hemodialysis and respiratory failure. There were no deaths in long-term follow-up., Conclusions: MAS is a complex vasculopathy with substantial variability in clinical presentation and anatomic distribution. Extensive disease frequently requires multiple invasive interventions and results in refractory hypertension, which may predict subsequent clinical events. A multidisciplinary approach with long-term monitoring is essential for preservation of end-organ function and quality of life in this debilitating disease., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Respiratory failure, the most frequent complication after thoracoabdominal aortic aneurysm (TAAA) repair, necessitates tracheostomy in severe cases. We examined risk factors for and outcomes of tracheostomy after TAAA repair., Methods: We reviewed the records of 1267 consecutive patients who underwent TAAA repair. Patients with a preexisting tracheostomy were excluded. Extensive repairs (Crawford extent I or II) were performed in 716 patients (56.6%). Stepwise logistic regression analysis was used to identify risk factors for postrepair tracheostomy., Results: Tracheostomy was necessary in 140 patients (11.1%). Operative mortality was significantly higher in patients with tracheostomy (27.9%) than in those without (5.8%; p < 0.001). As expected, tracheostomy patients had longer intensive care unit stays (24 vs 4 days, p < 0.001) and hospital stays (57 vs 10 days, p < 0.001) than nontracheostomy patients. Patients with tracheostomy were frequently transferred for additional long-term acute care or hospitalization (107, 76.4%), and many died after transfer (24/107, 22.4%). Kaplan-Meier curves showed markedly poorer late survival in patients with tracheostomy than in those without (47.9% ± 4.3% vs 87.3% ± 1.0% at 1 year; 27.8% ± 4.8% vs 68.6% ± 1.6% at 5 years). Independent predictors of post-TAAA repair tracheostomy included acute aortic dissection, chronic renal insufficiency, underweight body mass index, hypertension, history of stroke, extent II repair, diabetes, age at least 70 years, and greater platelet transfusion volume., Conclusions: Patients who undergo tracheostomy after TAAA repair have a high risk of early and late mortality as well as prolonged hospitalization. Strategies for improving survival outcomes in tracheostomy patients warrant investigation., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Acute kidney injury (AKI) necessitating renal replacement therapy adversely affects outcomes after thoracoabdominal aortic aneurysm (TAAA) repair. The effects of earlier stages of AKI are less known. We hypothesized that earlier stages of AKI would reduce early survival after TAAA repair., Methods: We analyzed prospectively collected data from 1,056 consecutive TAAA repairs from our institution (2006 to 2016). We excluded patients less than 18 years of age, those with preexisting renal disease, and three patients who died intraoperatively, resulting in 873 patients. The Kidney Disease Improving Global Outcomes criteria grouped patients into three AKI stages; stage 3 necessitated initiation of renal replacement therapy. Multivariable modeling identified operative mortality predictors. Kaplan-Meier analysis assessed 1-year survival., Results: Of 873 patients, 642 (73.5%) had no AKI and 231 (26.5%) had postoperative AKI (mild/stage 1, n = 92 [10.5%]; moderate/stage 2, n = 44 [5%]; severe/stage 3, n = 95 [10.9%]). Operative death occurred in 65 patients (7.4%): 14 (2.2%) with no AKI, 5 (5.4%) with mild AKI (p = 0.07 versus no AKI), 8 (18.2%) with moderate AKI (p = 0.02 versus mild), and 38 (40%) with severe AKI (p = 0.01 versus moderate). In multivariable analysis, moderate AKI independently predicted death (relative risk ratio: 9.4, 95% confidence interval: 3.4 to 25.9). Kaplan-Meier 1-year survival was 91.1% ± 1.2% for no AKI, 84.6% ± 3.9% for mild AKI (p = 0.07 versus no AKI), 67.4% ± 7.6% for moderate AKI (p = 0.01 versus mild), and 46.6% ± 5.3% for severe AKI (p = 0.02 versus moderate; p < 0.0001 across all groups)., Conclusions: Moderate/stage 2 AKI reduced early survival after TAAA repair. Prevention, earlier detection, and optimal medical management of AKI may improve survival., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Although reducing the incidence of unplanned readmission after thoracoabdominal aortic aneurysm (TAAA) repair represents an important opportunity to improve outcomes, predictors of readmissions are not known. We sought to characterize and identify factors associated with unplanned readmission after discharge in survivors of open TAAA repair., Methods: Through prospective phone contact and retrospective record review, we determined the frequency and characteristics of unplanned readmissions within 30 days of discharge in 363 patients who were discharged after open TAAA repair. We used univariate and multivariable analyses to identify factors associated with readmission., Results: There were 44 unplanned readmissions in 40 patients (11%). After readmission, 11 patients underwent operations, and 17 underwent nonsurgical procedures, the most common of which was thoracentesis (n = 9). Readmitted patients tended to have lower preoperative estimated glomerular filtration rates (p = 0.045), higher frequencies of preoperative sleep apnea (p = 0.009) and postoperative pulmonary (p = 0.04) and infection (p = 0.02) complications, and longer hospital stays (p = 0.01) than patients without readmissions. Patient age, urgency of operation, and extent of TAAA repair were similar in patients with and without readmissions. Multivariable analysis identified sleep apnea (relative risk ratio [RRR] 3.21, 95% confidence interval [CI]: 1.51 to 6.82, p = 0.002), postoperative infection (RRR 4.34, 95% CI: 1.32 to 14.25, p = 0.02), renal failure necessitating dialysis (RRR 3.14, 95% CI: 1.04 to 9.46, p = 0.04), and visceral artery stenting (RRR 2.43, 95% CI: 1.09 to 5.44, p = 0.03) as significant predictors of readmission., Conclusions: Patients with renal dysfunction, sleep apnea, or postoperative infection were particularly likely to be readmitted; optimizing the management of these factors may reduce early readmission after TAAA repair., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We analyzed trends, assessed outcomes and lessons learned, and investigated whether using a simplified US version of the frozen elephant trunk (FET) technique to treat complex arch pathology poses additional risk., Methods: From 2010 to 2015, we performed 129 consecutive ET procedures (traditional ET [t-ET], n = 92 [71.3%]; FET, n = 37 [28.7%]) for chronic dissecting (n = 62 [48.1%]) and atherosclerotic aneurysms (n = 67 [51.9%]). A stepwise logistic regression model using preoperative and intraoperative variables was created to analyze the outcomes., Results: Thirty-day mortality was 12.4% (t-ET, n = 9 [9.8%]; FET, n = 7 [18.9%]; p = 0.24). The rate of persistent (at the time of discharge) stroke was 5.4% (t-ET, n = 5 [5.4%]; FET, n = 2 [5.4%]; p =1.00). The rate of persistent spinal cord deficit was 3.9% (t-ET, n = 3 [3.3%]; FET, n = 2 [5.4%]; p = 0.62). In the multivariable analyses, the addition of FET was not an independent predictor of mortality, permanent stroke, or spinal cord deficit., Conclusions: With the advent of endovascular technology, there is a clinical shift toward increased use of FET to eliminate or facilitate the second surgical stage in treating patients with extensive aortic pathology. The addition of FET to the surgical armamentarium does not seem to pose additional risk (although larger studies are needed), but judicious use is advised nonetheless. A single-piece endoprosthesis for FET instead of a customized one should be considered., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Cardiac arrhythmias commonly arise after cardiac surgery and are associated with poor prognosis. In thoracoabdominal aortic aneurysm (TAAA) repair, these complications are poorly understood. We assessed characteristics, incidence, outcomes, and potential predictors of postoperative arrhythmia (PA) after open TAAA repair., Methods: From 2010 to 2014, 403 consecutive open TAAA replacement operations were performed in patients without preoperative cardiac rhythm abnormalities at a single tertiary center. We compared preoperative characteristics, operative factors, and postoperative outcomes in patients with and without PA, and we used multivariable logistic regression to identify predictors of PA., Results: PA occurred after 107 (26.5%) procedures. Atrial fibrillation (23%) was the most common type of PA. Length of hospital stay and operative mortality were greater in patients with PA than in patients without it (p < 0.01 for both). Kaplan-Meier cumulative survival for patients with PA was lower than for patients without PA: 69.2% ± 4.6% versus 88.3% ± 2.0% at 1 year and 59.0% ± 5.3% versus 85.0% ± 2.3% at 3 years (p < 0.001 for both). The odds of PA increased with advancing age (1.07 per year; p < 0.001). In addition, the odds of developing PA were higher in patients who received visceral perfusion (odds ratio, 2.58; p = 0.001) and were lower in patients who underwent extent IV repair (odds ratio, 0.44; p = 0.01)., Conclusions: Postoperative cardiac arrhythmia was common after open TAAA repair. Older patients and patients who underwent visceral perfusion were more likely to develop PA. Cardiac arrhythmia after TAAA repair was associated with prolonged hospital stay, higher early mortality, and lower midterm survival., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Evolving endovascular approaches to thoracoabdominal aortic aneurysm (TAAA) repair are attractive alternatives to the "gold standard" of conventional open TAAA repair. However, open repair may be more suitable for younger patients. We compared the outcomes of open TAAA repair in younger (≤50 years) and older (>50 years) patients to evaluate operative risk in younger patients., Methods: We analyzed retrospective and prospective data from 3,346 cases of open TAAA repair performed between 1986 and 2015. Of those patients, 445 (13.3%) were 50 years old or younger and 2,901 (86.7%) were older than 50 years at the time of repair. In the younger cohort, 237 patients (53.3%) had connective tissue disorder, and many (n = 359, 80.7%) had aortic dissection. Younger patients tended to be in good health, and rupture was uncommon (n = 11, 2.5%). The outcomes examined included adverse event, a composite endpoint that comprised operative death or persistent stroke, paraplegia, paraparesis, or renal failure requiring dialysis., Results: Comparisons between the two age groups showed that younger patients underwent more extent II repairs, urgent/emergent repairs, and visceral artery bypass procedures and had longer aortic clamp times. Nevertheless, younger patients had better early outcomes than did older patients, including lower rates of operative death (3.2% vs 8.2%, p = 0.002) and adverse events (5.2% vs 15.9%, p < 0.001). Multivariable analyses determined age 50 years old or younger to be an independent predictor of reduced adverse events (relative risk ratio = 0.29, p < 0.001)., Conclusions: Early outcomes of open TAAA repair were excellent for patients 50 years old or younger, despite the fact that these patients typically required extensive repairs., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: In patients with Marfan syndrome (MFS), distal aortic dissection can necessitate thoracoabdominal aortic aneurysm (TAAA) repair in survivors of acute DeBakey type I dissection and those with DeBakey type III dissection. We examined outcomes of surgical repair of TAAA in patients with MFS with distal aortic dissection., Methods: Data were analyzed for 127 consecutive TAAA repairs performed between January 2004 and June 2014 in patients with MFS and distal aortic dissection-DeBakey types I (n = 73) and III (n = 54). The median time from dissection onset to TAAA repair was 5.2 years (interquartile range [IQR]: 2.1 to 9.8 years) for the overall group and was longer in patients with DeBakey I (6.5 years, IQR: 3.5 to 13.9 years) than patients with DeBakey III (2.9 years, IQR: 0.6 to 6.0 years, p < 0.001). Eleven patients (9%) had acute or subacute dissection at the time of repair. Sixty-six patients (52%) underwent Crawford extent II TAAA repair. A composite end point, adverse event, was defined as operative death or permanent stroke, renal failure, paraplegia, or paraparesis., Results: Eight patients had adverse events (6%), including 5 operative deaths (4%). There was no permanent stroke and 1 case each of permanent paraplegia and paraparesis. At discharge, 2 early survivors (2%) had renal failure. Extent II repairs did not have substantially different outcomes from other repairs., Conclusions: In these patients with MFS with aortic dissection, open TAAA repair incurred reasonable operative risk, but improvements are needed to reduce rates of renal failure. Extent II TAAA repair does not appear to increase operative risk in patients with MFS., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Pathologic conditions of the aorta remain a major source of morbidity and mortality for patients with Marfan syndrome (MFS). Extensive thoracoabdominal aortic aneurysm (TAAA) repair can prevent aortic catastrophe but carries substantial risk of perioperative adverse events. We evaluated midterm survival and quality of life (QoL) after contemporary Crawford extent II TAAA repair in patients with MFS., Methods: From 2004 to 2010, 49 consecutive patients with MFS (mean age, 43.4 ± 12.0 years) underwent extent II TAAA repair (41 elective and 8 urgent/emergent procedures) with intercostal reimplantation. Thirty-six patients (73%) had aorta-related symptoms, and 45 (92%) had distal aortic dissection. Operative adjuncts included cerebrospinal fluid drainage (n = 47 [96%]), left heart bypass (n = 46 [94%]), and cold renal perfusion (n = 47 [96%]). Kaplan-Meier survival analysis was performed. QoL was assessed in 24 patients with a 12-item survey (12-Item Short Form Health Survey version 2 [SF-12v2]) a median of 5.3 (interquartile range [IQR], 4.0-7.9) years postoperatively. QoL data were normalized and compared with data from the general population., Results: There were no operative deaths, strokes, paraparesis, or paraplegia. Two patients (4%) had permanent renal failure necessitating hemodialysis. The most frequent complication was vocal cord paralysis (n = 21 [43%]). Six-year Kaplan-Meier survival was 84% ± 6%. The 24 patients with QoL data had slightly worse physical component scores (46.0 ± 10.6) and slightly better mental component scores (51.4 ± 10.4) than the general population (50 ± 10 for both scores)., Conclusions: Operative treatment of extensive TAAA in patients with MFS enables excellent midterm survival and QoL. Cerebrospinal fluid drainage, left heart bypass, and cold renal perfusion probably aid in achieving excellent outcomes., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We examined our contemporary experience with hemiarch and total arch replacement in patients with previous acute type I aortic dissection., Methods: Over an 8.5-year period, 137 consecutive patients (median age 58 years, interquartile range, 50 to 67) underwent hemiarch or total transverse aortic arch replacement a median of 7.7 years (range, 67 days to 32 years; interquartile range, 2.8 to 12.3 years) after previous acute type I aortic dissection repair. Interventions involving only the aortic root, aortic valve, descending aorta, or thoracoabdominal aorta were excluded. Multivariate analysis of 20 potential preoperative and intraoperative risk factors was performed to examine early death, neurologic deficit, composite endpoint (operative death, permanent neurologic deficit, or hemodialysis at discharge), and long-term mortality., Results: Total arch replacement was performed in 103 patients (75.2%), hemiarch replacement in 34 (24.8%), and elephant trunk procedures in 77 (56.2%). Thirty-one repairs (22.6%) were emergent or urgent. There were 16 operative deaths (11.7%), 4 permanent strokes (3.6%), and 21 (15.3%) instances of the composite endpoint. In the multivariate analysis, congestive heart failure and cardiopulmonary bypass time independently predicted operative mortality (p = 0.0027, p = 0.018). Emergency operation approached significance for stroke (p = 0.088). Predictors of long-term mortality (during a median follow-up period of 5.1 years, 95% confidence interval: 4.4 to 5.8) were female sex (p = 0.0036), congestive heart failure (p = 0.0045), and circulatory arrest time (p = 0.0013); preoperative pulmonary disease approached significance (p = 0.074). Five-year survival was 73.2%., Conclusions: In patients with previous acute type I aortic dissection repair, hemiarch and total arch operations have respectable morbidity and survival rates. Congestive heart failure predicts operative death, long-term mortality, and our adverse event endpoint. Cardiopulmonary bypass time predicts operative mortality, and female sex and circulatory arrest time predict long-term mortality., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Valve-sparing aortic root replacement (VSARR) is an alternative to traditional composite valve graft (CVG) root replacement. We examined early and midterm outcomes after VSARR., Methods: A combined retrospective/prospective study was performed in 83 patients who underwent VSARR (16%) among 515 patients who underwent aortic root replacement during a nearly 12-year period. Thirty-six patients (43%) had a connective tissue disorder, 3 patients (4%) had acute aortic dissection, and 40 (48%) patients had at least moderate aortic regurgitation (AR). Twenty-eight patients (34%) had left ventricular hypertrophy or dilatation. The reimplantation VSARR technique was used in 82 patients (99%), and the Florida sleeve technique was used in 1 patient. Thirty-two patients (39%) underwent concomitant aortic arch replacement. For early survivors, the median duration of follow-up was 3.5 years (range, 5 days-12.2 years)., Results: One patient had severe AR after VSARR that necessitated intraoperative conversion to a mechanical CVG. The 1 operative death and 1 stroke occurred in a patient with acute dissection. Actuarial survival was 96.4%±2.0% at 2 years and 86.9%±5.6% at 8 years. Six patients (7%) had late valve-related complications: 1 died of endocarditis, 4 underwent reoperation for severe AR and received replacement valves, and 1 had severe AR and is being monitored. Freedom from repair failure (reoperation, endocarditis, or severe AR) was 94.8%±2.6% at 2 years and 87.3%±5.7% at 8 years., Conclusions: Valve-sparing aortic root replacement can have excellent early and respectable midterm outcomes, even when combined with arch repair. Further follow-up remains necessary to evaluate the long-term durability of VSARR., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Aortic arch replacement remains among the most technically challenging cardiovascular operations, incurring considerable risk for perioperative death and stroke. The trifurcated graft technique, in which a double Y-graft is used to connect brachiocephalic branches to the main aortic graft, was recently developed to simplify arch reconstruction, reduce embolization, and minimize related cerebral ischemia. We examined early outcomes of aortic arch replacement performed by using single or double Y-graft variations of this technique., Methods: Between December 2006 and May 2009, the Y-graft technique was used to perform aortic arch replacement in 55 patients. Thirty-three patients had prior median sternotomy (60%), and 34 (62%) had ascending aortic dissection. Axillary cannulation was used in 52 patients (95%), and hypothermic circulatory arrest and antegrade cerebral perfusion were used in all patients. Median systemic and cerebral circulatory arrest times were 65 minutes and 0 minutes, respectively. A first-stage elephant trunk repair was performed in 46 patients (84%). Follow-up data were obtained for all patients., Results: There were no in-hospital deaths and one 30-day death (2%). Three patients (5%) had strokes, 1 of which was transient. Actuarial 1-year and 2-year survival rates were 80.0% ± 5.4% and 77.6% ± 5.7%, respectively. Thirty-one of the elephant trunk patients (67%) subsequently underwent second-stage completion procedures, 5 (16%) of them endovascular., Conclusions: Early results of aortic arch replacement by the Y-graft technique compare favorably with those of traditional approaches. The technique enables effective delivery of antegrade cerebral perfusion during complex arch procedures and incurs only a low risk of neurologic sequelae., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)